THE WHO 2000 TUMOR THE WHO 2000 TUMOR CLASSIFICATIONCLASSIFICATION

James G. Smirniotopoulos, M.D.James G. Smirniotopoulos, M.D.

Uniformed Services UniversityUniformed Services University

of the Health Sciencesof the Health Sciences

Bethesda, MDBethesda, MD

Visit us at: http://rad.usuhs.milVisit us at: http://rad.usuhs.mil

The WHO 2000The WHO 2000Classification ofClassification of

Brain TumorsBrain Tumors

DISCLAIMERDISCLAIMER::

The opinions expressed herein are those of the author(s), The opinions expressed herein are those of the author(s), and are not necessarily representative of the Uniformed and are not necessarily representative of the Uniformed Services University of the Health Sciences (Services University of the Health Sciences (USUHSUSUHS), the ), the Department of Defense (Department of Defense (DODDOD); or the World Health ); or the World Health Organization (Organization (WHOWHO). Medicine is a constantly changing ). Medicine is a constantly changing field, and medical information is subject to frequent field, and medical information is subject to frequent correction and revision. Therefore the reader is entirely correction and revision. Therefore the reader is entirely responsible for verifying the accuracy and relevance of the responsible for verifying the accuracy and relevance of the information contained herein. Portions copyright 1997 information contained herein. Portions copyright 1997 James G. Smirniotopoulos, M.D.James G. Smirniotopoulos, M.D.

Traditional Tumor Traditional Tumor GradingGrading

PATHOLOGISTPATHOLOGIST– LOW GRADELOW GRADE– HIGH GRADEHIGH GRADE

RADIOLOGISTRADIOLOGIST– NON-ENHANCINGNON-ENHANCING– ENHANCINGENHANCING

NEUROSURGEONNEUROSURGEON– ““SUCKABLE”SUCKABLE”– ““NON-SUCKABLE”NON-SUCKABLE”

Define the Problem:Define the Problem:

Some Low Grade EnhanceSome Low Grade Enhance Some Low Grade Do Not Some Low Grade Do Not

Some Low Grade => GBMSome Low Grade => GBM Some Low Grade Do NotSome Low Grade Do Not

WHO ClassificationWHO Classification

Defines Histologic SubtypesDefines Histologic Subtypes Grades Biologic PotentialGrades Biologic Potential Allows International Allows International

CooperationCooperation

WHO ClassificationWHO Classification

Biological PotentialBiological Potential

Ascending Scale of Aggression I - Ascending Scale of Aggression I - IVIV

WHO CORRELATIONWHO CORRELATION

Low GradeLow Grade

– Long-Term SurvivalLong-Term Survival

– Stable History (No Progression)Stable History (No Progression)

WHO GRADINGWHO GRADING

GRADE 1GRADE 1 JPAJPA SGCA SGCA GANG MENINGGANG MENING

GRADE 2GRADE 2 PXAPXA HPC HPC

GRADE 3GRADE 3 PXAPXA ANAPLASTIC ANAPLASTIC HPC HPC

GRADE 4GRADE 4 GBMGBM

CNS NEOPLASM-GLIALCNS NEOPLASM-GLIALPrognostic FactorsPrognostic Factors

LocationLocation AgeAge

HistologyHistology

MalignancyMalignancy

HISTOLOGIC MALIGNANCYHISTOLOGIC MALIGNANCY– microscopicmicroscopic

BIOLOGIC MALIGNANCYBIOLOGIC MALIGNANCY– macroscopicmacroscopic– labeling indiceslabeling indices– molecular biologymolecular biology

CLINICAL MALIGNANCYCLINICAL MALIGNANCY

HISTOLOGIC MALIGNANCYHISTOLOGIC MALIGNANCY

CELLULAR ATYPIACELLULAR ATYPIA MITOSES (Mitotic Index)MITOSES (Mitotic Index) INFILTRATION INFILTRATION

(lack of margination or (lack of margination or encapsulation)encapsulation)

NECROSISNECROSIS(sign of uncontrolled growth)(sign of uncontrolled growth)

VASCULAR CHANGESVASCULAR CHANGES(tumor neovascularity)(tumor neovascularity)

Kernohan-Sayre (AFIP)Kernohan-Sayre (AFIP)Grading System:Grading System:

GRADE IGRADE I - “BENIGN” or “Low-Grade”- “BENIGN” or “Low-Grade” GRADE II - “ “GRADE II - “ “ GRADE III - ANAPLASTICGRADE III - ANAPLASTIC

– cellular atypia, etc.cellular atypia, etc. GRADE IV- MALIGNANTGRADE IV- MALIGNANT

– NECROSIS !, Vascularity, MitosesNECROSIS !, Vascularity, Mitoses– GLIOBLASTOMA MULTIFORMEGLIOBLASTOMA MULTIFORME

NOTE: Numerous modifications exist, NOTE: Numerous modifications exist, most into three grades, e.g..: Low Grade most into three grades, e.g..: Low Grade (Benign), Anaplastic, and GBM (w/ (Benign), Anaplastic, and GBM (w/ NECROSIS).NECROSIS).

HIGH-GRADE ASTROCYTOMASHIGH-GRADE ASTROCYTOMASMEDIAN SURVIVAL:MEDIAN SURVIVAL:

ANAPLASTIC (Grade 3/4) - 28Mos.ANAPLASTIC (Grade 3/4) - 28Mos.

GBM (Grade 4/4, has NECROSIS) - GBM (Grade 4/4, has NECROSIS) - 8 Mos.8 Mos.

ASTROCYTOMAASTROCYTOMAFive Year SurvivalFive Year Survival

0 10 20 30 40 50 60 70 80 90 100

Percent Survival

Glioblastoma (IV)

Anaplastic (III)

Astrocytoma (I-II)

Pilocytic

BIOLOGIC BIOLOGIC MALIGNANCY:MALIGNANCY:

RAPID GROWTHRAPID GROWTH HEMORRHAGE, NECROSISHEMORRHAGE, NECROSIS LOCAL EXTENSIONLOCAL EXTENSION HEMATOGENOUS DISSEMINATIONHEMATOGENOUS DISSEMINATION

““BENIGN” ASTROCYTOMABENIGN” ASTROCYTOMA

Two types:Two types:–Low grade (“benign”)Low grade (“benign”)

DiffuseDiffuse (Adults)(Adults)–Low grade “special”Low grade “special”

Circumscribed Circumscribed (Children)(Children)

ASTROCYTIC ASTROCYTIC NEOPLASMSNEOPLASMS

WHO ClassificationWHO Classification Astrocytoma (Diffuse)Astrocytoma (Diffuse)(fibrillary, protoplasmic, or gemistocytic (fibrillary, protoplasmic, or gemistocytic

astrocytes)astrocytes) Anaplastic Astrocytoma (AA)Anaplastic Astrocytoma (AA) Glioblastoma Multiforme (GBM)Glioblastoma Multiforme (GBM)

(Giant Cell GBM, Gliosarcoma)(Giant Cell GBM, Gliosarcoma)____________________________________________________________

Pilocytic Astrocytoma (Juvenile - JPA)Pilocytic Astrocytoma (Juvenile - JPA) Subependymal Giant Cell AstrocytomaSubependymal Giant Cell Astrocytoma Superficial Cerebral AstrocytomaSuperficial Cerebral Astrocytoma Pleomorphic Xanthoastrocytoma (PXA)Pleomorphic Xanthoastrocytoma (PXA)

Grade vs. TypeGrade vs. TypeCircumscribed Diff use

StableHistology(Grade)

Pilocytic (1)SubependymalGiant Cell (1)

UnstableHistology(Grade)

PleomorphicXantho -astrocytoma (2- 3)

Astrocytoma (2)Anaplastic (3)GBM (4)

Enhancement vs. TypeEnhancement vs. TypeCircumscribed Diff use

NoEnhancement

Astrocytoma (2)

VariableEnhancement

Anaplastic (3)

RoutineEnhancement

Pilocytic (1)SEGCA(1)PXA (2- 3)

GBM (4)

PATTERN ANALYSISPATTERN ANALYSIS

Pilocytic(Juvenile)

Subependym alG iant

Cell Astrocytom a

Pleom orphicXanthoastrocytom a

Circum scribedAstrocytom a

ASTROCYTOMA:ASTROCYTOMA:CircumscribedCircumscribed

““Special” astrocytomas, Special” astrocytomas, Astrocytoma of YoungAstrocytoma of Young

Well circumscribed (yet, no capsule)Well circumscribed (yet, no capsule) Various LocationsVarious Locations Do NOT change grade (except PXA)Do NOT change grade (except PXA) Do NOT spread along WMDo NOT spread along WM Constellation of findings correlates Constellation of findings correlates

w/ Histologyw/ Histology

PILOCYTIC ASTROCYTOMAPILOCYTIC ASTROCYTOMA

Cystic Cerebellar Cystic Cerebellar Astrocytoma Juvenile Astrocytoma Juvenile Pilocytic AstrocytomaPilocytic Astrocytoma

(“PA” or “JPA”)(“PA” or “JPA”)

PILOCYTIC ASTROCYTOMAPILOCYTIC ASTROCYTOMA Synonyms: Polar Spongioblastoma, Cystic Synonyms: Polar Spongioblastoma, Cystic

Cerebellar AstrocytomaCerebellar Astrocytoma Cell of Origin: Astrocyte (bi-polar, hairlike)Cell of Origin: Astrocyte (bi-polar, hairlike) Associations: in ON w/ NF-1Associations: in ON w/ NF-1 Incidence: 3-6% of ALL Cranial, 32% of ChildIncidence: 3-6% of ALL Cranial, 32% of Child Age: 5-15 (Zulch 3-7) Sex: Slight F Age: 5-15 (Zulch 3-7) Sex: Slight F

(11/9)(11/9) Location: Cerebellum, Chiasm/Hypothal, Location: Cerebellum, Chiasm/Hypothal,

OpticOptic Treatment: Surgery, patienceTreatment: Surgery, patience Prognosis: 77% at 5 yrs.Prognosis: 77% at 5 yrs.

PILOCYTIC ASTROCYTOMAPILOCYTIC ASTROCYTOMARadiologyRadiology

Cerebellum, DiencephalonCerebellum, Diencephalon– rare in BS or Cerebrumrare in BS or Cerebrum

Majority have significant Majority have significant “cyst”“cyst”– ““Cyst and Mural Nodule”Cyst and Mural Nodule”

part of lining does NOT enhancepart of lining does NOT enhance

– Nodule may be heterogeneousNodule may be heterogeneous– Exceptional purely solidExceptional purely solid

Nodule NOT hyperdenseNodule NOT hyperdense Calcification in 5-25%Calcification in 5-25%

PATHOLOGYPATHOLOGY

Biphasic patternBiphasic pattern– dense pilocytic gliadense pilocytic glia– Rosenthal fibersRosenthal fibers– loose microcystic areasloose microcystic areas

No necrosisNo necrosis Low gradeLow grade Abnormal capillariesAbnormal capillaries

– allow enhancement, allow enhancement, fluidfluid

Grading GliomasGrading Gliomas51 51 Pilocytic (WHO Gr. 1)Pilocytic (WHO Gr. 1)

KERNOHAN KERNOHAN MAYO-ST.ANNE MAYO-ST.ANNE

1 26% 1 26% 1 2%1 2%

2 69%2 69% 2 55%2 55%

33 6% 6% 3 35%3 35%

4 0%4 0% 4 8%4 8%

ASTROCYTOMAASTROCYTOMAFive Year SurvivalFive Year Survival

0 20 40 60 80 100

Percent Survival

Glioblastoma (IV)

Anaplastic (III)

Astrocytoma (I-II)

Pilocytic

PILOCYTIC PILOCYTIC ASTROCYTOMA:ASTROCYTOMA:

LocationsLocations CEREBELLUMCEREBELLUM

Chiasm And Optic NerveChiasm And Optic Nerve

Hypothalmus/thalamusHypothalmus/thalamus Cerebral HemisphereCerebral Hemisphere Spinal Cord (Intramedullary)Spinal Cord (Intramedullary)

Pilocytic Astrocytoma Pilocytic Astrocytoma HemangioblastomaHemangioblastoma

Enhance Enhance EnhanceEnhance

Cyst w/ NoduleCyst w/ Nodule Solid <--> CysticSolid <--> Cystic

Hypodense noduleHypodense nodule HyperdenseHyperdense

Calcification Calcification Never Ca++Never Ca++

NOT vascular NOT vascular Hypervascular, Flow Hypervascular, Flow VoidsVoids

Nodule varies Nodule varies Nodule is “Subpial”Nodule is “Subpial”

PILOCYTIC ASTROCYTOMAPILOCYTIC ASTROCYTOMA(Juvenile Pilocytic)(Juvenile Pilocytic)

Childhood, Young AdultsChildhood, Young Adults Benign, no mitosis/necrosisBenign, no mitosis/necrosis Circumscribed - EnhancingCircumscribed - Enhancing Cyst Formation, Mural NoduleCyst Formation, Mural Nodule Cerebellum and DiencephalonCerebellum and Diencephalon

(Optic tracts, Hypothalmus)(Optic tracts, Hypothalmus)

WHO GRADE IWHO GRADE I

Circumscribed Circumscribed AstrocytomaAstrocytoma– JPA (Pilocytic)JPA (Pilocytic)– SGCA (Subependymal)SGCA (Subependymal)

GangliogliomaGanglioglioma MeningiomaMeningioma

CIRCUMSCRIBED vs CIRCUMSCRIBED vs DIFFUSEDIFFUSE

DiffuseAstrocytom a

Circum scribedAstrocytom a

Astrocytom a

NEUROECT ODERM ALNEOPLASM

ASTROCYTOMASASTROCYTOMAS

““SPECIAL” ASTROCYTOMASSPECIAL” ASTROCYTOMAS– Circumscribed Growth:Circumscribed Growth:

PilocyticPilocyticSubependymal Giant CellSubependymal Giant CellPleomorphic Xantho-Pleomorphic Xantho-AstrocytomaAstrocytoma

PLEOMORPHIC PLEOMORPHIC XANTHOASTROCYTOMAXANTHOASTROCYTOMA

Recently Described, Rare Recently Described, Rare Variant of AstrocytomaVariant of Astrocytoma

Arises from Subpial AstrocytesArises from Subpial Astrocytes Affects Superficial Cerebral Affects Superficial Cerebral

Cortex and MeningesCortex and Meninges Temporal > Frontal > ParietalTemporal > Frontal > Parietal

PLEOMORPHIC PLEOMORPHIC XANTHOASTROCYTOMAXANTHOASTROCYTOMA IMAGING:IMAGING:– CT APPEARANCE:CT APPEARANCE:

Well-Circumscribed Well-Circumscribed Hypodense or Cystic MassHypodense or Cystic Mass

Often Isodense Solid Nodule Often Isodense Solid Nodule That Intensely EnhancesThat Intensely Enhances

May Mimic Juvenile Pilocytic May Mimic Juvenile Pilocytic AstrocytomaAstrocytoma

Calcifications RareCalcifications Rare

PLEOMORPHIC PLEOMORPHIC XANTHOASTROCYTOMAXANTHOASTROCYTOMA

MR APPEARANCE:MR APPEARANCE:– Well-Circumscribed Mass of Variable Well-Circumscribed Mass of Variable

SizeSize– Superficial Cortical LocationSuperficial Cortical Location– T1: Low/Mixed Signal,T1: Low/Mixed Signal,– T2: High/Mixed SignalT2: High/Mixed Signal– Often with Cystic ComponentOften with Cystic Component– Solid Portion Intensely EnhancesSolid Portion Intensely Enhances– Adjacent Meninges May Enhance (Tail)Adjacent Meninges May Enhance (Tail)– Little or No Mass EffectLittle or No Mass Effect

ASTROCYTOMASASTROCYTOMAS

““ORDINARY” ASTROCYTOMAORDINARY” ASTROCYTOMADiffuse Infiltration of WM:Diffuse Infiltration of WM:

FibrillaryFibrillary ProtoplasmicProtoplasmic GemistocyticGemistocytic

WHO 2,3,4 (NOT 1)WHO 2,3,4 (NOT 1) KS & Mayo Grades 1-4KS & Mayo Grades 1-4

PATTERN ANALYSISPATTERN ANALYSISNeoplasmNeoplasm

Benign(G rade 1-2)(Fibrillary)

Anaplastic(G rade 2)

G lioblastom a(G rade 4)

DiffuseAstrocytom a

KERNOHAN (KS)KERNOHAN (KS) 11 2 2 3 3 4 4

ANAPLASIAANAPLASIA 0 0 MinMin >1/2 Marked >1/2 Marked

CELLULARITY CELLULARITY Mild Mild Mild IncMild Inc Marked Marked

MITOSISMITOSIS 0 0 0 0 Plus Marked Plus Marked

ENDOTHELIALENDOTHELIAL 0 Min0 Min Min Min Marked Marked

NECROSISNECROSIS Marked Marked

TRANSITIONTRANSITION<== Broad <== Broad Sharp ==> Sharp ==>

ST. ANNE-MAYOST. ANNE-MAYO (1-3) (1-3) 1 Point for 1 Point for Each Each

ATYPIAATYPIA

MITOSISMITOSIS

ENDOTHELIALENDOTHELIAL

NECROSISNECROSIS

Grade = TOTAL POINTSGrade = TOTAL POINTS

Grade 1 = 0-1, Grade 2 = 2 pointsGrade 1 = 0-1, Grade 2 = 2 points

Grade 3 = 3,4 pointsGrade 3 = 3,4 points

ASTROCYTOMA:ASTROCYTOMA:DIFFUSEDIFFUSE

(Fibrillary, protoplasmic, etc.)(Fibrillary, protoplasmic, etc.)

““Adult type” or “Hemispheric” Adult type” or “Hemispheric” AstrocytomaAstrocytoma

Diffusely infiltrate brain, along WM tractsDiffusely infiltrate brain, along WM tracts

Continuum, from low-grade to high-gradeContinuum, from low-grade to high-grade

Genetic Alterations 17 => 9 => 10Genetic Alterations 17 => 9 => 10

Many Progress in Histology over time, Many Progress in Histology over time, changing from WHO Gr. 2 => Gr. 3 => changing from WHO Gr. 2 => Gr. 3 => Gr. 4 (GBM)Gr. 4 (GBM)

Imaging tends to correlate with histologyImaging tends to correlate with histology

ASTROCYTOMAASTROCYTOMARadiologic GradingRadiologic Grading

TYPE 1 - (Benign, WHO 2 KS Grade 1-TYPE 1 - (Benign, WHO 2 KS Grade 1-2)2)– HomogeneousHomogeneous– No Enhancement, No EdemaNo Enhancement, No Edema

TYPE 2 - (Anaplastic - Grade 3)TYPE 2 - (Anaplastic - Grade 3)– Variable Enhancement, EdemaVariable Enhancement, Edema

TYPE 3 - (Glioblastoma - Grade 4)TYPE 3 - (Glioblastoma - Grade 4)– Heterogeneous (Necrosis, Blood)Heterogeneous (Necrosis, Blood)– Ring Enhancement, EdemaRing Enhancement, Edema

““BENIGN” BENIGN” ASTROCYTOMA:ASTROCYTOMA:

WHO 2, KS 1-2, Mayo 1WHO 2, KS 1-2, Mayo 1 YOUNGER PATIENTYOUNGER PATIENT

– CHILDHOODCHILDHOOD– Young Adults (20’s - 40’s)Young Adults (20’s - 40’s)

NL VESSELS (NO NL VESSELS (NO NEOVASCULARITY)NEOVASCULARITY)– BBB INTACTBBB INTACT– NO EDEMANO EDEMA– NO ENHANCEMENTNO ENHANCEMENT– NO TUMOR VESSELS NO TUMOR VESSELS

Benign - DiffuseBenign - Diffuse

HOMOGENEOUSHOMOGENEOUS– NO NECROSISNO NECROSIS– NO HEMORRHAGENO HEMORRHAGE– INCREASED WATERINCREASED WATER

DARK Poorly Demarcated on CTDARK Poorly Demarcated on CT

Sharp and Dark on T1W Sharp and Dark on T1W

Sharp and BRIGHT on T2WSharp and BRIGHT on T2W– MICROCYST >>> MACROCYSTMICROCYST >>> MACROCYST

(macrocysts occur in JPA, etc.)(macrocysts occur in JPA, etc.)

MODES OF SPREADMODES OF SPREAD

1. Natural passages1. Natural passages

2. Along surfaces2. Along surfaces

3. Along tracts3. Along tracts

4. Across the meninges4. Across the meninges

SPREAD ALONG SPREAD ALONG TRACTS:TRACTS:

CORONA RADIATACORONA RADIATA PEDUNCLESPEDUNCLES CORPUS CALLOSUMCORPUS CALLOSUM ANTERIOR COMMISUREANTERIOR COMMISURE ARCUATE FIBRESARCUATE FIBRES

““MALIGNANT” MALIGNANT” ASTROCYTOMA:ASTROCYTOMA: Older patientOlder patient

– 40’s and up40’s and up– exceptions (PNET)exceptions (PNET)– ~ 1/2 arise from previous low grade (2-3)~ 1/2 arise from previous low grade (2-3)

Abnl. Vessels (neovascularity)Abnl. Vessels (neovascularity) -- -- BBB abnormalityBBB abnormality– vasogenic edemavasogenic edema– contrast enhancementcontrast enhancement– irregular vessels, shunting, etc.irregular vessels, shunting, etc.

HETEROGENEOUSHETEROGENEOUS– hemorrhage (old/new)hemorrhage (old/new)– tumor necrosistumor necrosis– tumor itselftumor itself

GBMGBM

LOW DENSITY CENTERLOW DENSITY CENTER– variegated necrosisvariegated necrosis

ENHANCING RIMENHANCING RIM– hypercellular, fleshy neoplasmhypercellular, fleshy neoplasm– greatest neovascularitygreatest neovascularity

CORONA OF HYPODENSITYCORONA OF HYPODENSITY– ““edematous” white matteredematous” white matter– areas of neoplastic infiltrationareas of neoplastic infiltration

GLIOBLASTOMA GLIOBLASTOMA MULTIFORMEMULTIFORME

(Malignant Astrocytoma)(Malignant Astrocytoma)

Adults over 40 yrs.Adults over 40 yrs. Malignant with mitoses, Malignant with mitoses,

neovascularityneovascularity Discrete ring-enhancing lesionDiscrete ring-enhancing lesion Central necrosis, vasogenic edemaCentral necrosis, vasogenic edema Cerebral hemispheresCerebral hemispheres

(cross the corpus callosum)(cross the corpus callosum)

GLIAL TUMORSGLIAL TUMORSMR Grading*MR Grading*

Low Grade (KS 1-2)Low Grade (KS 1-2) High Grade (KS 3- High Grade (KS 3-4)4)

HomogeneousHomogeneous HeterogeneousHeterogeneous

Well definedWell defined Poorly definedPoorly defined

Min. MassMin. Mass More MassMore Mass

Min. Edema Min. Edema Vasogenic edemaVasogenic edema

No blood No blood HemosiderinHemosiderin

*Radiology (1990) 174: 411-415*Radiology (1990) 174: 411-415

GRADING SYSTEMSGRADING SYSTEMSSem Rad Onc (1991); 1: 2-9Sem Rad Onc (1991); 1: 2-9

KernohanKernohan BergerBerger WHOWHO1 1

Pilocytic,SEGAPilocytic,SEGA

Benign (1)Benign (1) AstrocytomaAstrocytoma

2 Astrocytoma2 Astrocytoma

Benign (2)Benign (2)

AnaplasticAnaplastic 3 Anaplastic3 Anaplastic

Anaplastic (3)Anaplastic (3)

GlioblastomaGlioblastoma 4 Glioblastoma4 Glioblastoma

Malignant (4)Malignant (4)

NEUROEPITHELIAL NEUROEPITHELIAL TUMORSTUMORS

WHO ClassificationWHO Classification AstrocyticAstrocytic OligodendroglialOligodendroglial EpendymalEpendymal Choroid Plexus TumorsChoroid Plexus Tumors NeuronalNeuronal Neuronal Mixed w/ GlialNeuronal Mixed w/ Glial PinealPineal Embryonal (PNET)Embryonal (PNET)

NEOPLASMS OF THE NEOPLASMS OF THE MENINGESMENINGES

WHO ClassificationWHO Classification

1. MENINGIOMA:1. MENINGIOMA:

a. Meningioma (typical)a. Meningioma (typical)

b. Atypical Meningiomab. Atypical Meningioma

c. Anaplastic (Malignant) c. Anaplastic (Malignant) MeningiomaMeningioma

2. MESENCHYMAL (non-meningothelial)2. MESENCHYMAL (non-meningothelial)

3. Primary MELANOCYTIC Lesions3. Primary MELANOCYTIC Lesions

4. UNCERTAIN Origin4. UNCERTAIN Origin

a. Hemangiopericytomaa. Hemangiopericytoma

b. Hemangioblastomab. Hemangioblastoma

MENINGEAL TUMORSMENINGEAL TUMORSWHO GradesWHO Grades

TYPETYPE GRADEGRADE

MENINGIOMAMENINGIOMA I I

ATYPICAL MENINGIOMAATYPICAL MENINGIOMA II II

PAPILLARY MENINGIOMAPAPILLARY MENINGIOMA II-IIIII-III

HEMANGIOPERICYTOMAHEMANGIOPERICYTOMA II-IIIII-III

ANAPLASTIC MENINGIOMAANAPLASTIC MENINGIOMA III III

MENINGIOMAMENINGIOMA“Malignant Meningioma”“Malignant Meningioma”

Hemangio-Peri-Cytoma (HPC)Hemangio-Peri-Cytoma (HPC) Malignant Fibrous Histiocytoma Malignant Fibrous Histiocytoma

(MFH)(MFH) Papillary MeningiomaPapillary Meningioma ““Benign” Metastasizing MeningiomaBenign” Metastasizing Meningioma

HEMANGIOPERICYTOMAHEMANGIOPERICYTOMA(HPC)(HPC)

Narrow dural baseNarrow dural base

(“Mushrooming”)(“Mushrooming”) No Hyperostosis, No No Hyperostosis, No

CalcificationCalcification Lobulated (not hemispheric)Lobulated (not hemispheric) Internal Signal Voids (on Internal Signal Voids (on

MRI)MRI) Hypervascular on AngioHypervascular on Angio

DYSEMBRYOPLASTIC DYSEMBRYOPLASTIC NEUROEPITHELIAL TUMOR (DNT)NEUROEPITHELIAL TUMOR (DNT)

IMAGING:IMAGING:– MR APPEARANCEMR APPEARANCE

Focal cortical mass, usually temporal Focal cortical mass, usually temporal lobelobe

Hypointense on T1Hypointense on T1 Hyperintense on T2Hyperintense on T2 MultinodularMultinodular

– MicrocysticMicrocystic– Megagyric - may cause bony erosionMegagyric - may cause bony erosion

Occasional EnhancementOccasional Enhancement

DYSEMBRYOPLASTIC DYSEMBRYOPLASTIC NEUROEPITHELIAL TUMOR (DNT)NEUROEPITHELIAL TUMOR (DNT)

IMAGING:IMAGING:– CT APPEARANCECT APPEARANCE

Hypodense MassHypodense Mass No EdemaNo Edema Rare CalcificationRare Calcification Calvarial ErosionCalvarial Erosion CT Normal in 10%CT Normal in 10%

THE NEW WHOTHE NEW WHO

Biological PotentialBiological Potential

Ascending Scale of Ascending Scale of AggressionAggression

I - IVI - IV

ASTROCYTOMAASTROCYTOMAFive Year SurvivalFive Year Survival

0 10 20 30 40 50 60 70 80 90 100

Percent Survival

Glioblastoma (IV)

Anaplastic (III)

Astrocytoma (I-II)

Pilocytic

CORRELATIONCORRELATION

Low GradeLow Grade

Long-Term Long-Term SurvivalSurvival

Stable HistologyStable Histology

WHO GRADE IWHO GRADE I

Circumscribed Circumscribed AstrocytomaAstrocytoma– JPA (Pilocytic)JPA (Pilocytic)– SGCA (Subependymal)SGCA (Subependymal)

GangliogliomaGanglioglioma MeningiomaMeningioma

Define the Problem:Define the Problem:

Some Low Grade EnhanceSome Low Grade Enhance Some Low Grade Do Not Some Low Grade Do Not

Some Low Grade => GBMSome Low Grade => GBM Some Low Grade Do NotSome Low Grade Do Not

Enhancement vs. TypeEnhancement vs. TypeCircumscribed Diff use

NoEnhancement

Astrocytoma (2)

VariableEnhancement

Anaplastic (3)

RoutineEnhancement

Pilocytic (1)SEGCA(1)PXA (2- 3)

GBM (4)